Amyloidosis, a tricky diagnosis, can be traced to Vietnam and exposure to Agent Orange

A hunch and multidisciplinary care sent advanced AL Amyloidosis into remission.
March 14, 2022
Steve Szymanski on his gravel bike, his battle with amyloidosis, a disease caused by exposure to Agent Orange, behind him... Photo courtesy of Steve Szymanski.
Steve Szymanski on his gravel bike, his battle with amyloidosis behind him. Photo courtesy of Steve Szymanski.

March being Amyloidosis Awareness Month, it’s a good time to tell the story of Steven Szymanski, who, until finally diagnosed with it, was roughly as familiar with this rare disease as you likely are.

One can be forgiven for not having heard of amyloidosis – though we’re all familiar with Alzheimer’s disease, its most common variety. Beyond the skull’s confines, the most prevalent form of systemic amyloidosis happens when blood-plasma cells gone rogue (cancers and other diseases cause this) make misshapen immune-system proteins. These misfolded light-chain immunoglobulin proteins link up with each other like the teeth of a zipper and create what Dr. Tomer Mark, a University of Colorado School of Medicine and UCHealth blood cancer specialist, described as “a sticky schmutz.”

“‘Amyloid’ derives from the Greek word for ‘starch,’” Mark said. “These proteins deposit themselves into organs, and eventually the organ becomes like a piece of plastic.”

Light-chain amyloidosis is the most common type of systemic amyloidosis. Also called AL amyloidosis, it occurs in 10% to 15% of the country’s roughly 35,000 annual multiple myeloma cases. Multiple myeloma is itself relatively rare – there are about nine times more new breast cancer cases each year. Still, the country’s roughly 4,000 annual AL amyloidosis additions would more than fill Denver’s Fillmore Auditorium.

Dr. Tomer Mark, a University of Colorado School of Medicine and UCHealth blood cancer specialist.
Dr. Tomer Mark, a University of Colorado School of Medicine and UCHealth blood cancer specialist.

The disease’s amyloid deposits can land in and ultimately destroy any organ. AL amyloidosis is most commonly diagnosed after the development of heart and kidney problems. When there is cardiac involvement, the disease can quickly be fatal.

Amyloidosis and Agent Orange

Szymanski, 74, of Centennial, is a retired real estate developer and avid cyclist whose run-in with amyloidosis can be traced back to his service in Vietnam. He spent 1969-1970 leading a platoon there, having been drafted after attending the University of Denver. The U.S. Veterans Administration provides health care and disability compensation for amyloidosis patients exposed to Agent Orange and other herbicides during their military service in Vietnam.

Szymanski was a serious cyclist, one fit enough to ride the 110-mile Triple Bypass into his 50s. He had never been treated in a hospital until 2013, when stabbing abdominal and lower-back pain of mysterious origins emerged. A series of tests – a lymph-node biopsy, a gallbladder biopsy, an endoscopy, a colonoscopy, and others – led to a diagnosis of celiac disease.

Szymanski has had to start over on his bike more than once. Among other motivations, two constant reminders: First, lots still to do in life. Second, it’s not forever. Photo courtesy of Steve Szymanski.
Szymanski has had to start over on his bike more than once. Among other motivations, two constant reminders: First, lots still to do in life. Second, it’s not forever. Photo courtesy of Steve Szymanski.

Cutting gluten made no difference. Szymanski arrived at UCHealth University of Colorado Hospital on the Anschutz Medical Campus (UCH) without a diagnosis. Szymanski’s wife Cyd estimates that they saw 15 doctors before meeting with UCHealth and CU School of Medicine internal-medicine physician Dr. Lisa Corbin. She referred Szymanski to gastrointestinal and other specialists, one of them being Dr. Kevin Deane, a CU School of Medicine and UCHealth colleague and rheumatologist.

The autoimmune-disease specialist ordered more tests. Deane noted high levels of protein in Szymanski’s urine – something previous tests had also found. He also ordered a blood test that looks for light-chain proteins. These queries would seem to have little to do with abdominal and back pain, but Deane’s experience as a rheumatologist had taught him to cast a wide net.

“Because our diseases are pretty rare, sometimes rheumatologists look for other things that mimic one of our diseases,” Deane said.

Stem-cell transplant

The light-chain protein test results convinced Deane that Szymanski would be best served by a hematologist. Before Deane even had the chance to meet Szymanski, he referred him to CU School of Medicine and UCHealth blood-cancer specialist Dr. Clay Smith. Smith soon diagnosed amyloidosis caused by multiple myeloma and coordinated a UCHealth care team including experts in cardiology, gastroenterology, infectious diseases and nephrology. The team found that amyloid deposits had damaged his kidneys and other abdominal organs. A heart fortified through a lifetime of strenuous exercise had also been hampered by plaques, a fact that its robustness had masked.

Dr. Clayton Smith, CU School of Medicine and UCHealth blood-cancer specialist.
Dr. Clayton Smith, CU School of Medicine and UCHealth blood-cancer specialist.

For Cyd, the diagnosis couldn’t have come sooner. She saw the swelling in her husband’s tongue and neck, the weight loss, and the purpura blood spots on his skin and under his eyes. He had been hospitalized for an abdominal hemorrhage. He was in pain.

“I could see that he was dying,” she said.

From March through June 2014, Szymanski went through 14 once-a-week rounds of chemotherapy. It was tough on him: A man who had gained just five pounds since college poured on 50 pounds of water weight from severe edema. He was used to suffering on a bike; this was different.

“I couldn’t walk 50 yards from my front door,” he said.

Szymanski with wife Cyd. Photo courtesy of Steve Szymanski.
Szymanski with wife Cyd. Photo courtesy of Steve Szymanski.

With time, he got better, and by late 2015, Szymanski seemed close to normal.

But in early 2016, the symptoms returned, and the treatment now would be more aggressive. Szymanski’s lifetime of exercise placed him among a select group of amyloidosis patients who can withstand an autologous hematopoietic stem-cell transplant.

This involves collecting bone marrow stem cells via filtering of the blood using a machine akin to dialysis. The machine separates the various cells in the blood, allowing for the collection of those cells capable of regeneration of a new bone marrow system, and delivers the rest of the blood back into the patient.

Once that process wraps up, the patient undergoes high-dose chemotherapy that destroys the bone marrow. The previously harvested stem cells are then introduced intravenously back to the patient’s body. The stem cells colonize the marrow and differentiate into a healthy and complete marrow system.

Stem cell transplants work because myeloma cells cannot live outside of the marrow, Mark says. Destroying the marrow and resupplying it with stem cells the next day is like “burning down a house to get rid of the termites and then building a new house the next day,” he says. As a result, the immune system gets “reset” as cells previously trained with vaccinations and prior infections now are replaced with fresh new cells.

“You grow a complexly new immune system. You get all your vaccinations again, just like a toddler,” Szymanski explained.

‘Still lots to do’

Dr. Paul Moroni, CU School of Medicine and UCHealth urologist.
Dr. Paul Moroni, CU School of Medicine and UCHealth urologist.

The stem-cell transplant made for a tough ride for two-and-a-half months, but Szymanski persevered. Mark took over his care and put him on low-dose, daily chemotherapy pill. Szymanski has continued it ever since, and amyloidosis and its triggering myeloma have remained in remission. His kidneys, which indeed sustained damage from the amyloid deposited prior to the stem-cell transplant, have finally recovered.

There have been other challenges, not least of which a prostate cancer diagnosis – another disease attributed to Agent Orange. CU School of Medicine and UCHealth urologist Dr. Paul Maroni has taken the lead on that treatment, which entailed eight weeks of radiation treatment starting in late 2017 and continuing with 18 months of hormone treatment.

Szymanski took time off the bike after his stem-cell treatment and during prostate-cancer hormone treatment that so knocked back his testosterone levels that, when he started riding again in 2018, “I was having to rebuild my cycling strength from scratch.”

He didn’t fret: His immune system had done something similar, after all. A note taped to his mirror for nearly a decade offered up two reminders:

1) Lots still to do in life.
2) It’s not forever.

Dr. Kevin Deane, CU School of Medicine and UCHealth colleague and rheumatologist.
Dr. Kevin Deane, CU School of Medicine and UCHealth colleague and rheumatologist.

Four years later, Moroni keeps an eye on Szymanski’s prostate cancer, and the patient is back on his bike. He rides 15 or more miles two or three times a week and lifts weights a couple of times a week to keep his strength up.

Szymanski extolls the efforts of Cyd as caretaker and patient advocate.

“Cyd has been front and center in this journey,” he said. “I don’t think I would have survived otherwise.”

Nor would he have without Deane’s intuition and the UCHealth team’s care, he adds.

“They saved my life,” he said.

About the author

Todd Neff has written hundreds of stories for University of Colorado Hospital and UCHealth. He covered science and the environment for the Daily Camera in Boulder, Colorado, and has taught narrative nonfiction at the University of Colorado, where he was a Ted Scripps Fellowship recipient in Environmental Journalism. He is author of “A Beard Cut Short,” a biography of a remarkable professor; “The Laser That’s Changing the World,” a history of lidar; and “From Jars to the Stars,” a history of Ball Aerospace.

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